Abstract

Carcinoid tumors are rare, but are the most common gastrointestinal (GI) neuroendocrine tumors. Within the GI tract, most carcinoids arise in the small intestine (45%, most commonly in the ileum), followed by rectum (20%), appendix (16%), colon (11%), and stomach (7%). PHCT is a very rare entity. Its description has been limited to case reports. We describe a case of PHCT and a review of the literature. Case: 49 year old Caucasian female with history of IBS and a negative abdominal CT 4 years earlier presents with abdominal pain, nausea and vomiting. Repeat imaging showed an 8.7 cm by 6.7 cm mass in the left lobe of the liver with a necrotic component. CT guided FNA was positive for Carcinoid Tumor. Extensive workup for a primary site, including an EGD, colonoscopy, UGI SBFT, and an octreotide scan were all negative. 24-hour urine 5 HIAA was normal. Serum serotonin was low. Patient underwent left hepatic lobectomy with clear margin of resection. Immunohistochemical staining showed strong reactivity to neoplastic cells for cytokeratin (AE1/3) and synaptophysin, and light to medium cytoplasmic staining for chromogranin. There was no reactivity for Hep-par-1 and CEA. There was nuclear staining for CDX-2. Repeat CT abdomen 2 years later shows no evidence of recurrent tumor. Discussion: In 2001, Iwao et al from Japan described a case report of PHCT and reviewed 53 previously published case reports. Analysis of these published cases indicates that PHCT occurs in the middle age (mean age = 48.2 years) and is more frequent in females (males/females = 20/33 cases). Of the symptomatic patients, the major findings were abdominal pain, fullness, and/or a palpable mass (56% of symptomatic patients). In contrast, only 2 cases out of 53 presented with symptoms of typical carcinoid syndrome. Surgical resection is the treatment primarily recommended with an 18% of recurrence rate and a 74% of a survival rate after 5 years. Treatment of inoperable cases has varied. Bastaki et at described percutaneous embolization of the tumor followed by complete dearterializations of the lesion. Knox et at performed a survival analysis with the Kaplan-Meier method on 48 published cases cited in the English literature. Actuarial 5- and 10-year survival for all patients was 78% and 59%, respectively, whereas for resected patients, 10-year survival was 68%. PHCT is a rare disorder. Diagnosis can be made by immunohistochemical staining and appropriate workup to exclude other primary sites. Treatment is surgical resection if technically feasible.

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